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Congenital retinal macrovessel associated with vitreous hemorrhage
Accepted Manuscript Congenital retinal macrovessel associated with vitreous hemorrhage Neha Goel, MS, DNB, FRCS (Glasg), Vinod Kumar, MS, DNB, FRCS (Glasg), Basudeb Ghosh, MD, MNAMS PII:
S1091-8531(16)30741-8
DOI:
10.1016/j.jaapos.2016.09.018
Reference:
YMPA 2539
To appear in:
Journal of AAPOS
Received Date: 4 May 2016 Revised Date:
15 September 2016
Accepted Date: 15 September 2016
Please cite this article as: Goel N, Kumar V, Ghosh B, Congenital retinal macrovessel associated with vitreous hemorrhage, Journal of AAPOS (2017), doi: 10.1016/j.jaapos.2016.09.018. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Congenital retinal macrovessel associated with vitreous hemorrhage Neha Goel, MS, DNB, FRCS (Glasg),a Vinod Kumar, MS, DNB, FRCS (Glasg),b Basudeb Ghosh, MD, MNAMSc
RI PT
Author affiliations: aICARE eye hospital and postgraduate institute, NOIDA, UP, India; bDr R. P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences (AIIMS), New Delhi, India; cGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India Submitted May 4, 2016. Revision accepted September 15, 2016.
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Correspondence: Neha Goel, 57, Sadar apartments, Mayur Vihar Phase 1 Extension, New Delhi – 110091, India (email: [email protected]).
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Word count: 860
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A “congenital retinal macrovessel” (CRM) is an aberrant retinal vessel (frequently a vein) that traverses the central macula and supplies or drains both above and below the horizontal raphe. It is an uncommon entity that is usually disclosed on routine examination
RI PT
and may rarely cause a compromised visual acuity. We describe the case of an adolescent girl who underwent vitrectomy for vitreous hemorrhage and was found to have a CRM with vascular abnormalities. To our knowledge, this is the first report of vitreous
SC
hemorrhage secondary to a CRM. Case Report
M AN U
A 15-year-old girl presented at Guru Nanak Eye Centre, New Delhi, with sudden loss of vision in her right eye of 1 month’s duration. There was no history of preceding trauma or flulike illness. On examination, her best-corrected visual acuity was counting fingers close to the face with accurate projection of rays in this eye. Anterior segment examination was within normal
TE D
limits, with an intraocular pressure (IOP) of 16 mm Hg using Goldmann applanation tonometry. An old, organized vitreous hemorrhage in the eye precluded a view of the fundus. The left eye was unremarkable, with a best-corrected visual acuity of 20/20.
EP
B-scan ultrasonography of the right eye revealed numerous low-intensity echoes in the vitreous cavity, Systemic investigations, including blood pressure, blood sugar, complete blood
AC C
counts, erythrocyte sedimentation rate, and C-reactive protein, were within normal limits. Tests for infective and autoimmune etiologies, including the Mantoux test, serum angiotensin converting enzyme levels, and tests for autoantibodies such as antinuclear antibody and rheumatoid factor, were negative. A 23-gauge pars plana vitrectomy was performed under general anesthesia. After clearing the hemorrhage, a dilated inferior retinal vein was identified (Figure 1A), and 3-5 rows of
ACCEPTED MANUSCRIPT
peripheral diode endolaser were applied. Postoperative treatment included topical antibioticsteroid combination, with cycloplegic drops. Best-corrected visual acuity was 20/60 on the first postoperative day, improving to 20/40
RI PT
at 1 month. Fundus fluorescein angiography (FFA) revealed a venous congenital retinal
macrovessel (CRM) with early filling and delayed emptying, with three branches crossing the horizontal raphe (Figure 1B). One of these tributaries passed through the macula but did not
SC
involve the fovea. In addition, hyperfluorescence from capillary telangiectatic vessels was present throughout the fundus (Figure 1C,D), including the macula. Laser marks applied
M AN U
intraoperatively were also noted (Figure 1D). There were no arteriovenous communications or capillary nonperfusion areas. Spectral domain optical coherence tomography of the macula demonstrated shadowing in the area of the CRM, with no other abnormalities. The patient was managed conservatively. Her clinical picture was maintained through 1 year of follow-up.
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Discussion
Congenital retinal macrovessel is a rare abnormality of the retinal circulation and is classified under group 1 of retinal arteriovenous malformations.4 Typically it is benign and clinically
EP
stable. Reduced vision, if present, has been attributed to hemorrhage, foveal cyst, the presence of the aberrant vessel in the fovea,1,2 or to central serous chorioretinopathy.3 CRM with decreased
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vision secondary to a branch retinal artery occlusion has also been reported.5 It seems that the retinal neural and vascular components develop independently of each other,6,7 thereby sparing the visual acuity in cases with CRM. This unusual vascular entity must be distinguished from prepapillary vascular loops, racemose hemangiomas, Wyburn-Mason syndrome, or other arteriovenous malformations with potential neuro-ocular sequelae.8 FFA in CRM demonstrates early filling with delayed emptying of the aberrant vessel
ACCEPTED MANUSCRIPT
associated with a dilated capillary bed surrounding the CRM.1 Areas of capillary nonperfusion, focal capillary dilatations, or direct arteriovenous communications may also be present.1,2,4 Macular small-vessel telangiectatic changes, as seen in our case, have been described
RI PT
previously1; however, distribution over such a large area of the fundus is a novel finding. It is possible that widespread small-vessel telangiectasia might have resulted in capillary
nonperfusion and resultant retinal neovascularisation may be the source of the vitreous
SC
hemorrhage in our patient. This was not demonstrable on fluorescein angiography, because intraoperative laser photocoagulation had already been carried out.
M AN U
Preretinal hemorrhage following activities that create a sudden increase in intraocular venous pressure (Valsalva retinopathy), with the rupture of capillary alterations, has been reported as a cause of decreased vision with CRM.2 Also, dramatic fluctuations in venous pressure superimposed on a CRM with arteriovenous communications has been reported to
TE D
induce decompensation of the CRM and neighboring microvasculature with consequential leakage and exudate formation.9 There was no history suggestive of Valsalva retinopathy in our case and no arteriovenous communications were visible on fluorescein angiography.
EP
Trauma is the leading cause of vitreous hemorrhage in children and adolescents, accounting for almost two-thirds of cases.10 The remainder, or “spontaneous” causes, include
AC C
Eales’ disease, sequelae of retinopathy of prematurity,10 and hematological disorders.11 There is no reported case of a CRM associated with spontaneous vitreous hemorrhage in children or adolescents.
A CRM is a rare, unilateral retinal vascular anomaly. This report adds to the handful of
cases in the literature where the presence of CRM is concomitant with a reduction in visual acuity. There is no previously reported case of vitreous hemorrhage associated with CRM.
ACCEPTED MANUSCRIPT
Literature Search An online search of the literature was performed using PubMed and Google using the following
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M AN U
SC
publications in English from 1956 to the present were reviewed.
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keywords in combination: congenital retinal macrovessel and vitreous hemorrhage. All available
ACCEPTED MANUSCRIPT
References 1.
Brown GC, Donoso LA, Margargal LE, Goldberg RE, Sarin LK. Congenital retinal macrovessels. Arch Ophthalmol 1982;100:1430-36. de Crecchio G, Alfieri MC, Cennamo G, Forte R. Congenital macular macrovessel. Graefes Arch Clin Exp Ophthalmol 2006;244:1183-7.
3.
RI PT
2.
Kumar V, Ghosh B, Raina U, Goel N. Central serous chorioretinopathy in a patient with
4.
Archer DB, Deutman A, Ernest JT, Krill AE. Arteriovenous communication of the retina.
M AN U
Am J Ophthalmol 1973;75:224-41. 5.
SC
congenital retinal macrovessel. Can J Ophthalmol 2009;44:e57.
Goel N, Kumar V, Seth A, Ghosh B. Branch retinal artery occlusion associated with congenital retinal macrovessel. Oman J Ophthalmol 2014;7:96-7. Ashton N. Retinal angiogenesis in the human embryo. Br Med Bull 1970;26:103-6.
7.
Volk D. Visual function studies in a case of large aberrant vessels in the macula. AMA
TE D
6.
Arch Ophthalmol 1956;55:119-22. 8.
Gurwood AS, Bailey JT, Pelino CJ. Congenital retinal macrovessel: a case report.
9.
EP
Optometry 2001;72:597-602.
Beatty S, Goodall K, Radford R, Lavin MJ. Decompensation of a congenital retinal
AC C
macrovessel with arteriovenous communications induced by repetitive rollercoaster rides. Am J Ophthalmol 2000;130:527-8.
10.
Rishi P, Rishi E, Gupta A, Swaminathan M, Chhablani J. Vitreous hemorrhage in children and adolescents in India. J AAPOS 2013;17:64-9.
11.
Sudhalkar A, Chhablani J, Jalali S, Mathai A, Pathengay A. Spontaneous vitreous hemorrhage in children. Am J Ophthalmol 2013;156:1267-71.
ACCEPTED MANUSCRIPT
Legends FIG 1. A, Fundus photograph of the right eye showing a dilated, tortuous inferotemporal retinal vein branching toward the central macula and draining above the horizontal raphe, suggestive of
RI PT
a venous congenital retinal macrovessel (CRM). B-D, Fundus fluorescein angiography. A venous CRM with draining venules (arrows) crossing the horizontal raphe (B) was confirmed, and capillary bed dilatation was noted, especially in the inferior part of the macula. Extensive
SC
capillary telangiectasia with mild leakage leading to hyperfluorescence was seen in the nasal fundus (C), with the inferior fundus also showing capillary telangiectasia with hyperfluorescence
AC C
EP
TE D
M AN U
and laser marks (D).
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
S1091-8531(16)30741-8
DOI:
10.1016/j.jaapos.2016.09.018
Reference:
YMPA 2539
To appear in:
Journal of AAPOS
Received Date: 4 May 2016 Revised Date:
15 September 2016
Accepted Date: 15 September 2016
Please cite this article as: Goel N, Kumar V, Ghosh B, Congenital retinal macrovessel associated with vitreous hemorrhage, Journal of AAPOS (2017), doi: 10.1016/j.jaapos.2016.09.018. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Congenital retinal macrovessel associated with vitreous hemorrhage Neha Goel, MS, DNB, FRCS (Glasg),a Vinod Kumar, MS, DNB, FRCS (Glasg),b Basudeb Ghosh, MD, MNAMSc
RI PT
Author affiliations: aICARE eye hospital and postgraduate institute, NOIDA, UP, India; bDr R. P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences (AIIMS), New Delhi, India; cGuru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India Submitted May 4, 2016. Revision accepted September 15, 2016.
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Correspondence: Neha Goel, 57, Sadar apartments, Mayur Vihar Phase 1 Extension, New Delhi – 110091, India (email: [email protected]).
AC C
EP
TE D
M AN U
Word count: 860
ACCEPTED MANUSCRIPT
A “congenital retinal macrovessel” (CRM) is an aberrant retinal vessel (frequently a vein) that traverses the central macula and supplies or drains both above and below the horizontal raphe. It is an uncommon entity that is usually disclosed on routine examination
RI PT
and may rarely cause a compromised visual acuity. We describe the case of an adolescent girl who underwent vitrectomy for vitreous hemorrhage and was found to have a CRM with vascular abnormalities. To our knowledge, this is the first report of vitreous
SC
hemorrhage secondary to a CRM. Case Report
M AN U
A 15-year-old girl presented at Guru Nanak Eye Centre, New Delhi, with sudden loss of vision in her right eye of 1 month’s duration. There was no history of preceding trauma or flulike illness. On examination, her best-corrected visual acuity was counting fingers close to the face with accurate projection of rays in this eye. Anterior segment examination was within normal
TE D
limits, with an intraocular pressure (IOP) of 16 mm Hg using Goldmann applanation tonometry. An old, organized vitreous hemorrhage in the eye precluded a view of the fundus. The left eye was unremarkable, with a best-corrected visual acuity of 20/20.
EP
B-scan ultrasonography of the right eye revealed numerous low-intensity echoes in the vitreous cavity, Systemic investigations, including blood pressure, blood sugar, complete blood
AC C
counts, erythrocyte sedimentation rate, and C-reactive protein, were within normal limits. Tests for infective and autoimmune etiologies, including the Mantoux test, serum angiotensin converting enzyme levels, and tests for autoantibodies such as antinuclear antibody and rheumatoid factor, were negative. A 23-gauge pars plana vitrectomy was performed under general anesthesia. After clearing the hemorrhage, a dilated inferior retinal vein was identified (Figure 1A), and 3-5 rows of
ACCEPTED MANUSCRIPT
peripheral diode endolaser were applied. Postoperative treatment included topical antibioticsteroid combination, with cycloplegic drops. Best-corrected visual acuity was 20/60 on the first postoperative day, improving to 20/40
RI PT
at 1 month. Fundus fluorescein angiography (FFA) revealed a venous congenital retinal
macrovessel (CRM) with early filling and delayed emptying, with three branches crossing the horizontal raphe (Figure 1B). One of these tributaries passed through the macula but did not
SC
involve the fovea. In addition, hyperfluorescence from capillary telangiectatic vessels was present throughout the fundus (Figure 1C,D), including the macula. Laser marks applied
M AN U
intraoperatively were also noted (Figure 1D). There were no arteriovenous communications or capillary nonperfusion areas. Spectral domain optical coherence tomography of the macula demonstrated shadowing in the area of the CRM, with no other abnormalities. The patient was managed conservatively. Her clinical picture was maintained through 1 year of follow-up.
TE D
Discussion
Congenital retinal macrovessel is a rare abnormality of the retinal circulation and is classified under group 1 of retinal arteriovenous malformations.4 Typically it is benign and clinically
EP
stable. Reduced vision, if present, has been attributed to hemorrhage, foveal cyst, the presence of the aberrant vessel in the fovea,1,2 or to central serous chorioretinopathy.3 CRM with decreased
AC C
vision secondary to a branch retinal artery occlusion has also been reported.5 It seems that the retinal neural and vascular components develop independently of each other,6,7 thereby sparing the visual acuity in cases with CRM. This unusual vascular entity must be distinguished from prepapillary vascular loops, racemose hemangiomas, Wyburn-Mason syndrome, or other arteriovenous malformations with potential neuro-ocular sequelae.8 FFA in CRM demonstrates early filling with delayed emptying of the aberrant vessel
ACCEPTED MANUSCRIPT
associated with a dilated capillary bed surrounding the CRM.1 Areas of capillary nonperfusion, focal capillary dilatations, or direct arteriovenous communications may also be present.1,2,4 Macular small-vessel telangiectatic changes, as seen in our case, have been described
RI PT
previously1; however, distribution over such a large area of the fundus is a novel finding. It is possible that widespread small-vessel telangiectasia might have resulted in capillary
nonperfusion and resultant retinal neovascularisation may be the source of the vitreous
SC
hemorrhage in our patient. This was not demonstrable on fluorescein angiography, because intraoperative laser photocoagulation had already been carried out.
M AN U
Preretinal hemorrhage following activities that create a sudden increase in intraocular venous pressure (Valsalva retinopathy), with the rupture of capillary alterations, has been reported as a cause of decreased vision with CRM.2 Also, dramatic fluctuations in venous pressure superimposed on a CRM with arteriovenous communications has been reported to
TE D
induce decompensation of the CRM and neighboring microvasculature with consequential leakage and exudate formation.9 There was no history suggestive of Valsalva retinopathy in our case and no arteriovenous communications were visible on fluorescein angiography.
EP
Trauma is the leading cause of vitreous hemorrhage in children and adolescents, accounting for almost two-thirds of cases.10 The remainder, or “spontaneous” causes, include
AC C
Eales’ disease, sequelae of retinopathy of prematurity,10 and hematological disorders.11 There is no reported case of a CRM associated with spontaneous vitreous hemorrhage in children or adolescents.
A CRM is a rare, unilateral retinal vascular anomaly. This report adds to the handful of
cases in the literature where the presence of CRM is concomitant with a reduction in visual acuity. There is no previously reported case of vitreous hemorrhage associated with CRM.
ACCEPTED MANUSCRIPT
Literature Search An online search of the literature was performed using PubMed and Google using the following
AC C
EP
TE D
M AN U
SC
publications in English from 1956 to the present were reviewed.
RI PT
keywords in combination: congenital retinal macrovessel and vitreous hemorrhage. All available
ACCEPTED MANUSCRIPT
References 1.
Brown GC, Donoso LA, Margargal LE, Goldberg RE, Sarin LK. Congenital retinal macrovessels. Arch Ophthalmol 1982;100:1430-36. de Crecchio G, Alfieri MC, Cennamo G, Forte R. Congenital macular macrovessel. Graefes Arch Clin Exp Ophthalmol 2006;244:1183-7.
3.
RI PT
2.
Kumar V, Ghosh B, Raina U, Goel N. Central serous chorioretinopathy in a patient with
4.
Archer DB, Deutman A, Ernest JT, Krill AE. Arteriovenous communication of the retina.
M AN U
Am J Ophthalmol 1973;75:224-41. 5.
SC
congenital retinal macrovessel. Can J Ophthalmol 2009;44:e57.
Goel N, Kumar V, Seth A, Ghosh B. Branch retinal artery occlusion associated with congenital retinal macrovessel. Oman J Ophthalmol 2014;7:96-7. Ashton N. Retinal angiogenesis in the human embryo. Br Med Bull 1970;26:103-6.
7.
Volk D. Visual function studies in a case of large aberrant vessels in the macula. AMA
TE D
6.
Arch Ophthalmol 1956;55:119-22. 8.
Gurwood AS, Bailey JT, Pelino CJ. Congenital retinal macrovessel: a case report.
9.
EP
Optometry 2001;72:597-602.
Beatty S, Goodall K, Radford R, Lavin MJ. Decompensation of a congenital retinal
AC C
macrovessel with arteriovenous communications induced by repetitive rollercoaster rides. Am J Ophthalmol 2000;130:527-8.
10.
Rishi P, Rishi E, Gupta A, Swaminathan M, Chhablani J. Vitreous hemorrhage in children and adolescents in India. J AAPOS 2013;17:64-9.
11.
Sudhalkar A, Chhablani J, Jalali S, Mathai A, Pathengay A. Spontaneous vitreous hemorrhage in children. Am J Ophthalmol 2013;156:1267-71.
ACCEPTED MANUSCRIPT
Legends FIG 1. A, Fundus photograph of the right eye showing a dilated, tortuous inferotemporal retinal vein branching toward the central macula and draining above the horizontal raphe, suggestive of
RI PT
a venous congenital retinal macrovessel (CRM). B-D, Fundus fluorescein angiography. A venous CRM with draining venules (arrows) crossing the horizontal raphe (B) was confirmed, and capillary bed dilatation was noted, especially in the inferior part of the macula. Extensive
SC
capillary telangiectasia with mild leakage leading to hyperfluorescence was seen in the nasal fundus (C), with the inferior fundus also showing capillary telangiectasia with hyperfluorescence
AC C
EP
TE D
M AN U
and laser marks (D).
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT